Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2016 Sep 15;5(9):e003289.
doi: 10.1161/JAHA.116.003289.

Coronary Artery Aneurysms in Kawasaki Disease: Risk Factors for Progressive Disease and Adverse Cardiac Events in the US Population

Kevin G Friedman  1 , Kimberly Gauvreau  2 , Akiko Hamaoka-Okamoto  3 , Alexander Tang  3 , Erika Berry  4 , Adriana H Tremoulet  4 , Vidya S Mahavadi  4 , Annette Baker  2 , Sarah D deFerranti  2 , David R Fulton  2 , Jane C Burns  5 , Jane W Newburger  2
Affiliations

Coronary Artery Aneurysms in Kawasaki Disease: Risk Factors for Progressive Disease and Adverse Cardiac Events in the US Population

Kevin G Friedman et al. J Am Heart Assoc. .

Abstract

Background: The natural history of coronary artery aneurysms (CAA) after intravenous immunoglobulin (IVIG) treatment in the United States is not well described. We describe the natural history of CAA in US Kawasaki disease (KD) patients and identify factors associated with major adverse cardiac events (MACE) and CAA regression.

Methods and results: We evaluated all KD patients with CAA at 2 centers from 1979 to 2014. Factors associated with CAA regression, maximum CA z-score over time (zMax), and MACE were analyzed. We performed a matched analysis of treatment effect on likelihood of CAA regression. Of 2860 KD patients, 500 (17%) had CAA, including 90 with CAA z-score >10. Most (91%) received IVIG within 10 days of illness, 32% received >1 IVIG, and 27% received adjunctive anti-inflammatory medications. CAA regression occurred in 75%. Lack of CAA regression and higher CAA zMax were associated with earlier era, larger CAA z-score at diagnosis, and bilateral CAA in univariate and multivariable analyses. MACE occurred in 24 (5%) patients and was associated with higher CAA z-score at diagnosis and lack of IVIG treatment. In a subset of patients (n=132) matched by age at KD and baseline CAA z-score, those receiving IVIG plus adjunctive medication had a CAA regression rate of 91% compared with 68% for the 3 other groups (IVIG alone, IVIG ≥2 doses, or IVIG ≥2 doses plus adjunctive medication).

Conclusions: CAA regression occurred in 75% of patients. CAA z-score at diagnosis was highly predictive of outcomes, which may be improved by early IVIG treatment and adjunctive therapies.

Keywords: Kawasaki disease; cardiovascular outcomes; coronary aneurysm.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Patient selection. CAA indicates coronary artery aneurysm; CHD, congenital heart disease; IVIG, intravenous immunoglobulin; KD, Kawasaki disease; MACE, major adverse cardiac events.
Figure 2
Figure 2
Patient outcomes. CA indicates coronary artery; CAA, coronary artery aneurysm; CABG, coronary artery bypass graft; MI, myocardial infarction; PCI, percutaneous coronary artery intervention.
Figure 3
Figure 3
Kaplan‐Meier curve for coronary artery aneurysm regression: entire cohort. CAA indicates coronary artery aneurysm.
Figure 4
Figure 4
Kaplan‐Meier curves for coronary artery aneurysm regression. A, Decade of Kawasaki disease episode. B, IVIG treatment within 10 days of fever onset. C, Maximum coronary artery z‐score at diagnosis. D, Location of coronary artery aneurysm. CAA indicates coronary artery aneurysm; IVIG, intravenous immunoglobulin; LAD, left anterior descending coronary artery; RCA, right coronary artery.
See this image and copyright information in PMC

References

    1. Kawasaki T. [Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children]. Arerugi. 1967;16:178–222. - PubMed
    1. Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC, Shulman ST, Bolger AF, Ferrieri P, Baltimore RS, Wilson WR, Baddour LM, Levison ME, Pallasch TJ, Falace DA, Taubert KA; Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association . Diagnosis, treatment, and long‐term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Pediatrics. 2004;114:1708–1733. - PubMed
    1. Burns JC, Glode MP. Kawasaki syndrome. Lancet. 2004;364:533–544. - PubMed
    1. McCrindle BW, Li JS, Minich LL, Colan SD, Atz AM, Takahashi M, Vetter VL, Gersony WM, Mitchell PD, Newburger JW; Pediatric Heart Network I . Coronary artery involvement in children with Kawasaki disease: risk factors from analysis of serial normalized measurements. Circulation. 2007;116:174–179. - PubMed
    1. Terai M, Shulman ST. Prevalence of coronary artery abnormalities in Kawasaki disease is highly dependent on gamma globulin dose but independent of salicylate dose. J Pediatr. 1997;131:888–893. - PubMed

MeSH terms

Substances